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Pulmonary alveolar proteinosis

Nature and origin of alveolar lipid
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      Abstract

      The alveolar material and the metabolism of pulmonary lipids in alveolar proteinosis was studied. We analyzed pulmonary tissue from three patients and pulmonary washings from nine patients with alveolar proteinosis in various phases of the disorder, and investigated synthesis and dissipation of pulmonary lipids. The results were compared with similar observations in patients with other pulmonary diseases.
      Phospholipid, principally palmitoyl lecithin, was the predominant lipid in tissue and washings from both groups of patients. The total lipid and phospholipid content of tissue and washings was much greater in patients with active alveolar proteinosis than in patients without this disorder, but the relative proportions of phosphatide fractions and fatty acid esters were similar in both groups of patients. The lipid normally present in lung washings and that accumulating in alveolar proteinosis is the same and it is synthesized by the lung. No enhancement of lipid synthesis could be demonstrated in alveolar proteinosis but the removal or degradation of alveolar phospholipid was found to be impaired. There was no deficiency of plasma lipoprotein lipase activity. Pulmonary washings contained increased amounts of protein, but this protein did not have electrophoretic mobility and was apparently denatured. It contained large amounts of palmitoyl lecithin but the material accumulating within alveoli did not have surface tension-lowering activity and was inhibitory to surfactant. The concept is advanced that alveolar proteinosis results from prolonged retention of the metabolic products and by-products of the alveolar lining.
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      References

        • Rosen S.H.
        • Castleman B.
        • Liebow A.A.
        Pulmonary alveolar proteinosis.
        New England J. Med. 1958; 258: 1123
        • Ray R.L.
        • Salm R.
        A fatal case of pulmonary alveolar proteinosis.
        Thorax. 1962; 17: 257
        • Stansifer P.D.
        • Bourgeois C.
        Pulmonary alveolar proteinosis: histochemical observations.
        Am. J. Clin. Path. 1965; 44: 539
        • Taxay E.P.
        • Montgomery R.D.
        • Wildish D.M.
        Studies of pulmonary alveolar microlithiasis and pulmonary alveolar proteinosis.
        Am. J. Clin. Path. 1960; 24: 532
        • Larson R.K.
        • Gordinier R.
        Pulmonary alveolar proteinosis: reports of six cases, review of the literature, and formulation of a new theory.
        Ann. Int. Med. 1965; 62: 292
        • Ramirez J.
        • Schultz R.B.
        • Dutton R.E.
        Pulmonary alveolar proteinosis. A new technique and rationale for treatment.
        Arch. Int. Med. 1963; 112: 419
        • Ramirez J.
        • Kieffer Jr., R.F.
        • Ball Jr., W.C.
        Bronchopulmonary lavage in man.
        Ann. Int. Med. 1965; 63: 819
        • Ramirez J.
        Bronchopulmonary lavage. New techniques and observations.
        Dis. Chest. 1966; 50: 581
        • Ramirez J.
        • Campbell G.D.
        Pulmonary alveolar proteinosis: endobronchial treatment.
        Ann. Int. Med. 1965; 63: 429
        • Ramirez J.
        Pulmonary alveolar proteinosis. Treatment by massive bronchopulmonary lavage.
        Arch. Int. Med. 1967; 119: 147
        • Ramirez J.
        Pulmonary alveolar proteinosis. Treatment of a case complicated by tuberculosis.
        Am. Rev. Resp. Dis. 1967; 95: 491
        • Hawkins J.E.
        • Savard E.
        • Ramirez-Rivera J.
        Pulmonary alveolar proteinosis: origin of protein in pulmonary washings.
        Am. J. Clin. Path. 1967; 48: 14
        • Said S.I.
        • Avery M.E.
        • Davis R.K.
        • Banarjee C.M.
        • El-Gohary M.
        Pulmonary surface activity in induced pulmonary edema.
        J. Clin. Invest. 1965; 44: 458
        • Kuenzig M.E.
        • Hamilton R.W.
        • Peltier L.F.
        Dipalmitoyl lecithin: studies on surface properties.
        J. Appl. Physiol. 1965; 20: 779
        • Bressler R.
        • Katz R.I.
        The role of carnitine in acetoacetate production and fatty acid synthesis.
        J. Clin. Invest. 1965; 44: 840
        • Trout D.L.
        • Estes Jr., E.H.
        • Friedberg S.J.
        Tritration of free fatty acids of plasma: a study of current methods and a new modification.
        J. Lipid Res. 1960; 1: 199
        • Lees R.S.
        • Hatch F.T.
        Sharper separation of lipoprotein species by paper electrophoresis in albumin-containing buffer.
        J. Lab. & Clin. Med. 1963; 61: 518
        • Folch J.
        • Lees M.
        • Sloane-Stanley G.H.
        A simple method for the isolation and purification of total lipids from animal tissues.
        J. Biol. Chem. 1957; 226: 497
        • Marinetti G.B.
        • Albrecht M.
        • Ford T.
        • Stotz E.
        Analysis of human plasma phosphatides by paper chromatography.
        Biochim. et biophys. acta. 1959; 36: 4
        • Abell L.L.
        • Levy B.B.
        • Brodie B.B.
        • Kendall F.E.
        A simplified method for the estimation of total cholesterol in serum and demonstration of its specificity.
        J. Biol. Chem. 1952; 195: 357
        • Van Handel E.
        • Zilversmidt D.B.
        Micromethod for the direct determination of serum triglycerides.
        J. Lab. & Clin. Med. 1957; 50: 152
        • Harlan Jr., W.R.
        • Margraf J.H.
        • Said S.I.
        Pulmonary lipid composition of species with and without surfactant.
        Am. J. Physiol. 1966; 211: 855
        • Harlan Jr., W.R.
        • Wakil S.J.
        Synthesis of fatty acids in animal tissues. I. Incorporation of C14-acetyl coenzyme A into a variety of long-chain fatty acids by subcellular particles.
        J. Biol. Chem. 1963; 238: 3216
        • Harlan Jr., W.R.
        • Said S.I.
        • Banarjee C.M.
        Metabolism of pulmonary phospholipids in normal lung and during pulmonary edema.
        Am. Rev. Resp. Dis. 1966; 94: 938
        • Fredrickson D.S.
        • Ono K.
        • Davis L.L.
        Lipolytic activity of postheparin plasma in hyperglyceridemia.
        J. Lipid Res. 1963; 4: 24
        • Heinemann H.O.
        Free fatty acid production by rabbit lung tissue in vitro.
        Am. J. Physiol. 1961; 201: 607
        • Zipl R.E.
        • Waldo A.L.
        Spectrophotometric analysis of carbohydrates and study of anthrone reagent.
        J. Lab. & Clin. Med. 1952; 39: 497
        • Clements J.A.
        • Hustead R.F.
        • Johnson R.P.
        • Gribetz I.
        Pulmonary surface tension and alveolar stability.
        J. Appl. Physiol. 1963; 18: 665
        • Harlan Jr., W.R.
        • Winesett P.S.
        • Wasserman A.J.
        Tissue lipoprotein lipase in normal individuals and in individuals with exogenous hypertriglyceridemia and the relationship of this enzyme to assimilation of fat.
        J. Clin. Invest. 1967; 46: 239
        • Klaus M.
        • Reiss O.
        • Tooley W.H.
        • Piel C.
        • Clements J.A.
        Alveolar epithelial cells mitochondria as a source of surface-active lung lining.
        Science. 1962; 137: 750
        • Bensch K.
        • Schaefer K.
        • Avery M.E.
        Granular pneumocytes: electron microscopic evidence of their exocrinic function.
        Science. 1964; 145: 1318
        • Kuhn C.
        • Gyorkey F.
        • Levine B.E.
        • Ramirez-Rivera J.
        Pulmonary alveolar proteinosis. A study using enzyme histochemistry, electron microscopy and surface tension measurement.
        Lab. Invest. 1966; 15: 492
        • Nicholas J.J.
        • Auchincloss Jr., J.H.
        • Rudolph L.
        Pulmonary alveolar proteinosis: a case with improvement after a short course of endobronchial instillations of heparin.
        Ann. Int. Med. 1965; 62: 358
        • Vogel W.C.
        • Bierman E.L.
        Post-heparin serum lecithinase in man and its positional specificity.
        J. Lipid Res. 1960; 8: 46